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June 1966

Retinopathy in a Case of Farber's Lipogranulomatosis

Author Affiliations

From the Howe Laboratory of Ophthalmology, Harvard University Medical School; Massachusetts Eye and Ear Infirmary; and the pediatric and neurology services, Massachusetts General Hospital, Boston.

Arch Ophthalmol. 1966;75(6):752-757. doi:10.1001/archopht.1966.00970050754007

A syndrome characterized by progressive hoarseness, nodular swellings over the joints simulating rheumatoid arthritis, and death in early infancy was described by Farber and colleagues in 1957 under the heading of "disseminated lipogranulomatosis."1 It is currently being reviewed by Crocker et al.2 The entity has come to be known as Farber's disease since the term "lipogranulomatosis" is not sufficiently distinctive and since Farber had established priority by referring to it in previous discussions of the lipidoses.3

One of Farber's original three cases was noted to have been blind, but the fundi showed no abnormality and no histologic study was made of the eyes postmortem. In the other two cases of the entity which have been reported,4,5 no mention has been made of any ocular abnormality in the retina or choroid.

The present report is based on the clinical and pathologic study of a patient who was